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• W3142926997 abstract "Since Professor WANG Zhong-gao's creative work on the systemic treatment of the Budd-Chiari syndrome (BCS),1 this debilitating disease has been more and more widely recognized in China. Several large-scale studies of surgery or intervention strategies for treating BCS have been reported.1-4 However, much controversy still remains regarding many aspects of this disease, including its etiology, treatment, and classification. This review explores these controversies with emphasis on areas that merit further study. ETIOLOGY OF BCS Regional difference in the incidence of BCS is poorly understood. A relatively high incidence of the BCS has been observed in developing countries such as China, India, Nepal, and South Africa.5 Domestically, an increased incidence of BCS has been reported for the mid-downstream areas of the Yellow River and the drainage basin of the Huaihe River, including the provinces of Shandong, Henan, Northern Anhui, Hebei, and in the city of Xuzhou of the province of Jiangsu. A relatively lower incidence of BCS has been reported in Western countries, and most cases suffer acute thrombosis of the vena cava and/or the hepatic veins as a result of hypercoagulability, which may be related to racial differences or the use of contraceptive drugs.6 In contrast, most domestic cases of BCS appear to be associated with excessive membranous structures within the inferior vena cava (IVC) and/or the hepatic veins (HVs).5 While it is often assumed that these membranous lesions are congenital in origin, the exact mechanisms underlying their formation remain unknown, even in spite of numerous epidemiological studies.5 Interestingly, we showed in a retrospective study of 60 BCS cases that 70% (42/60 cases) showed signs of membrane formation within the IVC. Moreover, almost all of these patients with membranous lesions were more than 20 years old, with an average age of 33. The exceptional case was an 11-year-old patient. These observations are not consistent with a congenital origin of membranous formation, since congenital occlusion of the IVC and/or HVs would be expected to show symptoms of BCS during childhood. Nonetheless, there have been no published cases of children with BCS to date. Evidence exists that formation of membranous occlusions can occur during adulthood. We have treated two recurrent cases of BCS in which newly formed membrane was detected within the IVC, although it was not observed during surgery one year prior. Thus one year may be sufficient time for the formation of membrane. Moreover, a case of recurrent membrane formation following its repeated surgical removal has been reported.7 While these reports are certainly suggestive, other corroborating evidence is currently lacking. This may be due to the fact that few domestic vascular surgeons have the resources to apply direct radical surgery in the treatment of BCS. Most cases are treated with endovascular therapy or shunting procedures, and, membranous lesions can only be diagnosed through direct radical surgery. Not surprisingly, many cases of BCS with acquired membrane occlusions may not have been correctly diagnosed. Cases with short segmental occlusions of the IVC may actually be due to membranous lesions. The supra-membranous segment of the IVC may be adhesive because of negative pressure between the membrane and the right atrium. Pathological studies have shown the membrane to be a fibrous tissue, sometimes with hyaline degeneration similar to an organized clot. Through our experience with 60 cases of radical surgery we believe we have observed a transformation from thrombus to membrane in the IVC and mural thrombus of the IVC to membranous lesions of the HVs. We therefore suggest that formation of membranous occlusions of IVC or HVs are an acquired disease rather than a congenital one. Further research is now being undertaken to examine this issue. Another area that merits investigation is the relationship between BCS and infection. BCS is more common in poor areas, among people lacking appropriate health care, and is rare in urban centers. In fact, evidence of parasitic infection near a lesion has been reported.8 TREATMENT Conventional treatment for BCS mainly consists of four different kinds of open surgery: 1) indirect decompression procedures including ascites drainage from peritoneal cavity to the jugular vein, thoracic duct to jugular vein anastomosis, devascularization procedures, and other operations to enhance collateral circulation such as splenopneumopexy; 2) direct decompression, such as cavoatrial, mesoatrial, mesojugular, splenoatrial, mesocaval, splenocaval, or portocaval shunts; 3) direct surgical correction of lesions; 4) liver transplantation. Among the first category of surgical procedures, thoracic duct to jugular vein anastomosis and other operations to enhance collateral circulation have now been almost completely abandoned because of poor efficacy. Ascites drainage from peritoneal cavity to the jugular vein actually has no benefit for BCS and is only occasionally applied as a transitional operation for cases with such a poor general condition that they cannot tolerate more invasive procedures. Ultimately, radical surgery or decompression procedures must be performed, upon improvement of the general condition. Direct decompression procedures, while less surgically complicated, are the most frequently applied conventional surgical procedures. Mesocaval and portocaval shunts are now rarely used due to high rates of hepatic coma. The most commonly used shunting procedures, including cavoatrial, mesoatrial, mesojugular, and splenoatrial shunts, have shown acceptable short-term effects in the clinical setting. However, the long-term effects are concerning because of the high rate of recurrence and the need for repeated procedures due to prosthetic thrombosis.9 Radical surgery has a major advantage, repairing the IVC anatomically and physiologically and avoiding severe complications such as hepatic coma. We have recently treated a case with occluded hepatic veins and with a large amount of ascites (nearly 30 000 ml released intraoperatively), umbilical hernia, and uterine prolapse. Following radical surgery, the patient recovered quickly without complication. We have performed novel surgical techniques to expose the entire IVC of the hepatic segment for 60 patients with BCS. Both the short and long-term effects of novel surgery were relatively satisfactory, with only one death and one restenosis without caval patching. However, radical surgery has not been widely applied to the treatment of BCS, likely because most surgeons consider the second hepatic portal as a forbidden operating area because of its complicated anatomy. We have managed to develop a novel procedure by which the IVC of the hepatic segment can be satisfactorily exposed. First, the intrapericardial IVC is exposed by opening the pericardial sac, and an incision is made in the diaphragm from the bare area of the liver towards the IVC to expose the IVC of the retrohepatic segment. Another incision is made in the distal part of the diaphragm to expose the IVC just above the right kidney. Finally the middle segment of the IVC (retrohepatic IVC), which is most tightly adhesive to the liver, can be completely and easily exposed because the location of the middle-segment of the IVC can be discerned with the reference to the exposed proximal and distal IVC. Autotransfusion of blood through the right atrial cannulation with a cardiopulmonary bypass machine makes it unnecessary to dissect the HVs and helps greatly to reduce the operation time. This procedure greatly reduces the risk of excessive bleeding. We are usually able to perform the entire procedure within 2 to 3 hours, a much shorter time period than that of most shunting procedures. Liver transplantation is mainly applied in cases with end-stage hepatic failure or diffuse occlusion in hepatic veins or veinules. However, operation and post-operation expenses are relatively high and the long-term results of liver transplantation are still unknown.10 Besides conventional open surgeries, various minimally invasive methods for BCS treatment have emerged with the development of the endovascular therapy. Endovascular therapy is the first choice for membranous lesions of BCS because of its minimal invasiveness. The first successful treatment of membranous occlusion of IVC with percutaneous transfemoral vein mem-branectomy was reported in 1974 by Eguchi et al.11 Subsequently, Yamada et al12 treated a patient with a segmental IVC occlusion with percutaneous transfemoral vein balloon angioplasty in 1983. Endovascular therapy for BCS mainly includes the following procedures: 1) percutaneous transfemoral vein membranotomy and balloon angioplasty of the IVC; 2) percutaneous transfemoral vein membranotomy and balloon angioplasty plus stenting of the IVC; 3) antegrade percutaneous transhepatic recanalization of the HVs; 4) antegrade percutaneous transhepatic recanalization plus stenting of the HVs; 5) percutaneous transjugular vein recanalization of the HVs; 6) percutaneous transjugular vein recanalization plus stenting of the HVs; 7) transfemoral vein catheterization for IVC thrombolysis; 8) transfemoral vein retrograde recanalization of the HVs. Endovascular therapy for the accessory HVs also belongs to this group of procedures. On the basis of endovascular therapy we have developed semi-interventional treatment for BCS including a combination of endovascular therapy with open surgery and a combination of additional open procedures with primary interventional treatment.13,14 Semi-interventional treatment is more invasive than pure interventional treatment but much less invasive than conventional open surgery. Thus Semi-interventional treatment may be the first choice for those failed cases after interventional treatment and difficult cases for interventional treatment. Semi-interventional treatment mainly includes the following procedures: 1) combined transatrial and transfemoral vein membranotomy and dilation of the IVC; 2) combined transatrial and transfemoral vein membranotomy and dilation plus stenting of the IVC; 3) radical resection of the IVC lesions plus stenting; 4) interventional treatment for the IVC lesions combined with additional surgeries such as ascites drainage operation from peritoneal cavity to the jugular vein, mesocaval shunt, mesoatrial shunt, or mesojugular shunt. Endovascular therapy may be the first choice for short segmental occlusion of the IVC and removal of membrane within the IVC and HVs. In the case of unsuccessful interventional treatment, combined membranotomy may be advisable. Radical surgery may be a better choice for cases with IVC thrombosis or membrane occlusion with distal IVC thrombosis for surgeons who have the resources to perform this procedure. However, based on our experience, bypass and shunting procedures are less effective, have poor long-term effects, and are therefore not worth pursuing. Finally, liver transplantation may be the only treatment for patients with end-stage liver failure and diffused occlusion of hepatic veins or veinules. CLASSIFICATION Many categories of BCS have been established to instruct clinical practices. The first classification system in China, put forward by Professor WANG Zhong-gao, included eight types: type I, membranous occlusion of the IVC with a foramen, also called membranous stenosis; type II, completely membranous occlusion of the IVC; type III, short segmental stenosis of the IVC; type IV, short segmental occlusion of the IVC; type V, long segmental stenosis of the IVC; type VI, long segmental occlusion of the IVC; type VII, occlusion near the opening of the HVs; type VIII, diffused occlusion of the HVs. Dr. XU Pei-qin4 classified the BCS into four types and six subtypes: type Ia, membranous occlusion of the IVC without infra-membranous thrombosis and with patent or partly patent main HVs; type Ib, membranous occlusion of the IVC with infra-membranous thrombosis and with patent or partly patent main HVs; type II, segmental stenosis of the IVC and segmental occlusion of the HVs; type IIIa, segmental (<2 cm) occlusion of the IVC with occlusion of main HVs and compensated expansion of the right posterior HV; type IIIb, segmental (>2 cm) occlusion of the IVC with occlusion of main HVs and without expansion of the third portal of the IVC; type IV, any of the above types combined with stenosis or occlusion of the superior vena cava. Interventional doctors have also developed classifications based on radial or image characteristics of the BCS. Finally, Dr. ZU Mao-heng has classified BCS into four types and eight subtypes: type I, membranous lesions of the IVC, including two subtypes with or without foramen; type II, segmental lesions of the IVC, including two subtypes with stenosis or occlusion; type III, lesions of the HVs, including two subtypes with or without accessory HVs; type IV, mixed, with lesions of the IVC and HVs, including two subtypes with or without accessory HVs. None of these classification systems is without shortcomings. Through our experience with radical surgery, we have not found any bona fide stenosis of the IVC itself, and what might be assumed to be a stenosis has proven to be the result of compression from the hepatomegaly caused by occlusion of the HVs. In fact, we have treated several hundred cases of BCS since 1992 and not a single case of long segmental stenosis of the IVC was among them. Long segmental stenosis of the IVC is more probably caused by mural thrombosis of the IVC, which results in occlusion of the outlet of the HVs. We therefore believe that the BCS with IVC stenosis category should be eliminated from all BCS classification systems. Instead we suggest a new simple classification system that includes three types and six subtypes, based on lesions in the IVC and/or the HVs: type I, lesions of the IVC including three subtypes: Ia membranous lesion, Ib short segmental occlusion (<5 cm), Ic long segmental occlusion (>5 cm); type II, lesions of the HVs including two subtypes with membranous lesions or diffuse occlusion; type III, mixed type with lesions of the IVC and the HVs." @default.
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• W3142926997 date "2007-01-01" @default.
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• W3142926997 title "Etiology, treatment, and classification of Budd-Chiari syndrome" @default.
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